PCK2 - phosphoenolpyruvate carboxykinase 2, mitochondrial Gene

Also Known as PEPCK; PEPCK2; PEPCK-M

Species: Homo sapiens

Gene Type: protein coding
Gene ID: 5106

About PCK2

Cytogenetic location: 14q11.2-q12 Genomic coordinates (GRCh38): 14:24,094,171-24,104,125 (from NCBI)

This gene has 16 transcripts (splice variants), 1 gene allele, 201 orthologues, 1 paralogue and is associated with 2 phenotypes. Biased expression in duodenum (RPKM 127.7), small intestine (RPKM 91.2) and 8 other tissues.

Summary

This gene encodes a mitochondrial enzyme that catalyzes the conversion of oxaloacetate to phosphoenolpyruvate in the presence of guanosine triphosphate (GTP). A cytosolic form of this protein is encoded by a different gene and is the key enzyme of gluconeogenesis in the liver. Alternatively spliced transcript variants have been described. [provided by RefSeq, Apr 2014]

PCK2 Products (4)

mRNA Protein Name
NM_001018073.3 NP_001018083.2 phosphoenolpyruvate carboxykinase [GTP], mitochondrial isoform 2 precursor
NM_001291556.2 NP_001278485.1 phosphoenolpyruvate carboxykinase [GTP], mitochondrial isoform 3
NM_001308054.2 NP_001294983.1 phosphoenolpyruvate carboxykinase [GTP], mitochondrial isoform 3
NM_004563.4 NP_004554.3 phosphoenolpyruvate carboxykinase [GTP], mitochondrial isoform 1 precursor

Gene Ontology

  • Molecular Function
  • Cellular Component
Molecular Function GO Annotation Evidence References Source
enables manganese ion binding IDA
IDA: Inferred from direct assay
28955899 GOA
enables phosphoenolpyruvate carboxykinase (GTP) activity EXP
EXP: Inferred from Experiment
9657976 GOA
enables phosphoenolpyruvate carboxykinase (GTP) activity IDA
IDA: Inferred from direct assay
28955899 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
21988832 GOA
Cellular Component GO Annotation Evidence References Source
located in mitochondrion IDA
IDA: Inferred from direct assay
8645161 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

PCK2 Protein Structure

PEPCK_C

PEPCK_C: Phosphoenolpyruvate carboxykinase C-terminal P-loop domain (46 - 639)

  • 0
  • 100
  • 200
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  • 400
  • 500
  • 600
  • 640 a.a.
Protein Preferred Names Protein Names

phosphoenolpyruvate carboxykinase [GTP], mitochondrial

  • PEP carboxykinase

PCK2 Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method References
Intra
PCK2 Q16822 SMAD3 Homo sapiens P84022
Y2H
21988832
Intra
PCK2 Q16822 SMAD3 Homo sapiens P84022
Anti Tag CoIP
21988832
Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant PCK2 Proteins

Cat. No. Product Name Accession Purity
HY-P75957 PCK2 Protein, Human (sf9, His-GST) Q16822-1/NP_004554.3 (L33-M640) ≥ 90%, as determined by reducing SDS-PAGE.

PCK2 Antibodies

Cat. No. Product Name Application Reactivity
HY-P81802 PCK2 Antibody (YA1547) WB, IHC-F, IHC-P, ICC/IF Human, Mouse, Rat
HY-P81802A PCK2 Antibody (YA1547)(PBS only) WB, IHC-F, IHC-P, ICC/IF Human, Mouse, Rat
HY-P84621 PCK2 Antibody (YA4318) WB, IHC-P, ICC/IF, FC, ELISA Human, Rat, Monkey
HY-P84621A PCK2 Antibody (YA4318)(PBS only) WB, IHC-P, ICC/IF, FC, ELISA Human, Rat, Monkey
HY-P85451 PCK2 Antibody (YA5143) WB Human, Mouse, Rat, Bovine, Dog, Pig

Related Diseases

Diseases Alias
Phosphoenolpyruvate Carboxykinase Deficiency, Mitochondrial

  • Pck2 Deficiency

  • Phosphoenolpyruvate Carboxykinase 2 Deficiency

  • PCKDM

  • Pepck2 Deficiency

  • Pepck Deficiency, Mitochondrial

  • Pepck 2 Deficiency

  • Pepck2

  • Mitochondrial Phosphoenolpyruvate Carboxykinase Deficiency

  • M-PEPCKD
Pepck 1 Deficiency

  • Phosphoenolpyruvate Carboxykinase Deficiency

  • Phosphoenolpyruvate Carboxykinase Deficiency

  • Pep Carboxykinase Deficiency

  • Phosphoenolpyruvate Carboxykinase-1 Deficiency

  • Phosphoenolpyruvate Carboxylase Deficiency

  • Phosphopyruvate Carboxylase Deficiency

  • Pepck Deficiency
Hypoglycemia

  • Hypoglycaemia

  • Low Blood Sugar

  • Hypoglycaemia Nos

  • Spontaneous Hypoglycaemia

  • Nondiabetic Hypoglycaemia

  • Hypoglycaemic Disorder Nos

  • Hypoglycaemic Syndrome
Glucose Intolerance

  • Glucose: Intolerance

  • Glucose: Malabsorption

  • Malabsorption Of Glucose

  • Impaired Glucose Tolerance
Hyperglycemia
Type 2 Diabetes Mellitus

  • Insulin Resistance

  • NIDDM

  • Type 2 Diabetes

  • Diabetes Mellitus, Non-Insulin-Dependent

  • T2D

  • Noninsulin-Dependent Diabetes Mellitus

  • Diabetes Mellitus, Type Ii

  • Maturity-Onset Diabetes

  • Insulin Resistance, Severe, Digenic

  • Diabetes Mellitus, Type 2

  • Diabetes Mellitus, Noninsulin-Dependent

  • Diabetes Mellitus, Noninsulin-Dependent, Association With

  • Diabetes Mellitus, Noninsulin-Dependent, Late Onset

  • Hypertension, Insulin Resistance-Related, Susceptibility To

  • Insulin Resistance, Susceptibility To

  • Non-Insulin-Dependent Diabetes Mellitus

  • Type Ii Diabetes Mellitus

  • Adult-Onset Diabetes Mellitus

  • Maturity-Onset Diabetes Mellitus

  • Diabetes Mellitus Type 2

  • Type Ii Diabetes

  • Type 2 Diabetes Mellitus, Susceptibility To

  • Diabetes, Type 2

  • Diabetes Mellitus, Noninsulin-Dependent, Susceptibility To

  • Diabetes Mellitus, Non-Insulin-Dependent, Susceptibility To

  • Diabetes Mellitus, Type 2, Susceptibility To

  • Diabetes Mellitus, Noninsulin-Dependent, 2

  • Diabetes Mellitus, Type Ii, Susceptibility To

  • Hypertension, Insulin Resistance-Related

  • Adult-Onset Diabetes

  • Aodm

  • Diabetes Mellitus, Adult-Onset

  • Diabetes Mellitus Type Ii

  • Diabetes Mellitus Type 2, Susceptibility To

  • Diabetes, Type Ii, Susceptibility To

  • Diabetes Type 2

  • Diabetes Mellitus

  • Adult Onset Diabetes

  • Maturity Onset Diabetes

  • Nonketotic Diabetes

  • Non-Insulin Dependent Diabetes Mellitus

  • T2dm - [Type 2 Diabetes Mellitus]

  • Niddm - [Non Insulin Dependent Diabetes Mellitus]

  • Dm2

  • Dm Type Ii

  • Diabetic Type 2

  • Insulin Requiring Type 2 Diabetes

  • Noninsulin Dependent Diabetes

  • Non-Insulin-Dependent Diabetes Mellitus Without Complications

  • Diabetes Due To Insulin Secretory Defect

  • Diabetes Mellitus Due To Insulin Secretory Defect

  • Non-Insulin-Dependent Diabetes Of The Young

  • Senile Diabetes

  • Nonketotic Hyperglycaemia

  • Stable Diabetes
Maturity-Onset Diabetes Of The Young

  • MODY

  • Maturity Onset Diabetes Mellitus In Young

  • Mason-Type Diabetes

  • Mason Type Diabetes

  • Maturity Onset Diabetes Of The Young

  • Mody Syndrome

  • Diabetes Of The Young, Maturity-Onset
Acute Porphyria

  • Porphyrias, Hepatic

  • Hepatic Porphyria

  • Porphyria Hepatic

  • Acute Intermittent Porphyria
Lactic Acidosis

  • Acidosis, Lactic

  • Acidosis Lactic
Diabetes Mellitus

  • Diabetes
Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Related Agents

  • Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (3)
Cat. No. Product Name
Effect
Purity
Rare Diseases
HY-RS10153 PCK2 Human Pre-designed siRNA Set A Pre-designed Set / Unkown
HY-RS16227 Pck2 Mouse Pre-designed siRNA Set A Pre-designed Set / Unkown
HY-RS23789 Pck2 Rat Pre-designed siRNA Set A Pre-designed Set / Unkown

Orthologs Information

Species Symbol Source ID
Felis catus PCK2 VGNC VGNC:68727
Rattus norvegicus PCK2 RGD RGD:1311112
Canis familiaris PCK2 VGNC VGNC:44308
Macaca mulatta PCK2 VGNC VGNC:75780
Bos taurus PCK2 VGNC VGNC:32632
Mus musculus PCK2 MGD MGI:1860456
Others PCK2 NCBI